A case of myxopapillary ependymoma of the lateral ventricle is reported. The patient was a 30 year old woman who had headache for 9 months' duration & visual disturbance of left eye for 2 months' duration. Brain CT and MRI scan revealed solid mass at trigonal area of left lateral ventricle with cystic component. And cerebral angiogram revealed faint punctate mottled staining. Total removal of the tumor was done via transcortical transventricular approach without further neurologic deficit. The tumor was diagnosed as myxopapillary ependymoma with H & E staining, immunohistochemical staining for gliofibrillary acidic protein and electron microscopic examination, Postoperative radiotherapy was performed on primary area & whole brain field with the dose of 5500 rads.